The protein encoded by this gene, liver glycogen synthase, catalyzes the rate-limiting step in the synthesis of glycogen - the transfer of a glucose molecule from UDP-glucose to a terminal branch of the glycogen molecule. Mutations in this gene cause glycogen storage disease type 0 (GSD-0) - a rare type of early childhood fasting hypoglycemia with decreased liver glycogen content. [provided by RefSeq, Dec 2009]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtGlycogen storage disease 0

Clinical highlights

Gene-disease validity (ClinGen)
glycogen storage disorder due to hepatic glycogen synthase deficiency · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
24
Pubs (1 yr)
P/LP submissions
P/LP missense
1.13
LOEUF
DN
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.13LOEUF
pLI 0.000
Z-score 0.89
OE 0.85 (0.651.13)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.14Z-score
OE missense 0.98 (0.901.07)
374 obs / 381.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.85 (0.651.13)
00.351.4
Missense OE?0.98 (0.901.07)
00.61.4
Synonymous OE?1.19
01.21.6
LoF obs/exp: 36 / 42.2Missense obs/exp: 374 / 381.6Syn Z: -1.79

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GYS2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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